PLoS Pathog. 2025 Feb 4;21(2):e1012784. doi: 10.1371/journal.ppat.1012784. Online ahead of print.

ABSTRACT

Aspergillus fumigatus (Af) is a major mould pathogen found ubiquitously in the air. It commonly infects the airways of people with cystic fibrosis (CF) leading to Aspergillus bronchitis or allergic bronchopulmonary aspergillosis. Resident alveolar macrophages and recruited neutrophils are important first lines of defence for clearance of Af in the lung. However, their contribution to the inflammatory phenotype in CF during Af infection is not well understood. Here, utilising CFTR deficient mice we describe a hyperinflammatory phenotype in both acute and allergic murine models of pulmonary aspergillosis. We show that during aspergillosis, CFTR deficiency leads to increased alveolar macrophage death and persistent inflammation of the airways in CF, accompanied by impaired fungal control. Utilising CFTR deficient murine cells and primary human CF cells we show that at a cellular level there is increased activation of NFκB and NFAT in response to Af which, as in in vivo models, is associated with increased cell death and reduced fungal control. Taken together, these studies indicate that CFTR deficiency promotes increased activation of inflammatory pathways, the induction of macrophage cell death and reduced fungal control contributing to the hyper-inflammatory of pulmonary aspergillosis phenotypes in CF.

PMID:39903773 | DOI:10.1371/journal.ppat.1012784

Pediatr Pulmonol. 2024 Jun;59(6):1661-1676. doi: 10.1002/ppul.26970. Epub 2024 Apr 12.

ABSTRACT

BACKGROUND: Cystic fibrosis (CF) is a life-shortening multisystem genetic disease. Although progressive pulmonary disease is the predominant cause of morbidity and mortality, improvements in treatment for CF-related lung disease, with associated increase in longevity, have increased the prevalence of extrapulmonary manifestations1.

METHODS: To discuss these issues, a multidisciplinary meeting of international leaders and experts in the field was convened in November 2021 at the Shaping Initiatives and Future Trends Symposium with the goal of highlighting shifting management paradigms in CF. The main topics covered were: (1) nutrition and obesity, (2) exocrine pancreas, (3) CF-related diabetes, (4) CF liver disease, (5) CF-related bone disease, and (6) post-lung transplant care. This document summarizes the proceedings, highlighting the key priorities and important research questions that were discussed.

RESULTS: Improved life expectancy, the advent of cystic fibrosis transmembrane conductance regulator modulators, and the increasing appreciation of the heterogeneity or spectrum of disease are leading to a shift in management for patients with cystic fibrosis. Care should be individualized to ensure that increased longevity is accompanied by improved extra-pulmonary care and reduced morbidity.

PMID:39903130 | DOI:10.1002/ppul.26970

Health Qual Life Outcomes. 2025 Feb 4;23(1):10. doi: 10.1186/s12955-025-02338-2.

ABSTRACT

BACKGROUND: Measuring the quality of life in patients with cystic fibrosis is important, both in terms of assessing the implementation of new therapies and monitoring their effects, as well as the ongoing evaluation of patients' condition. The objective of this study is to present tools for measuring the quality of life in adult patients with cystic fibrosis, along with their characteristics and measurement properties.

METHODS: The systematic review was performed according to the PRISMA guidelines based on a previously prepared research protocol (PROSPERO: CRD42023491030). Searches were performed in Medline (via PubMed), Embase (via OVID), and Cochrane Library databases. In addition, manual searches of bibliographies from the studies included in the analysis and grey literature were performed. Quality assessment of the included studies was performed according to the guidelines of COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN).

RESULTS: The systematic search identified 3,359 studies, of which 26 met the inclusion criteria for the analysis. Two publications were additionally included as a result of the manual search. A total of 16 tools for measuring the quality of life in adults with cystic fibrosis were identified, the measurement properties of which were presented in the included studies. Among these tools, the Cystic Fibrosis Questionnaire-Revised (CFQ-R) and the Cystic Fibrosis Quality of Life Questionnaire (CFQoL) were most frequently analyzed. There were also other new, promising tools.

CONCLUSION: Most studies reported acceptable measurement properties of tools used to measure quality of life in adult patients with cystic fibrosis. In many cases, however, significant limitations were observed related to the lack of comprehensive analysis of the factor structure and other aspects related to validation and responsiveness. There have also been problems with the reliability of some tool scales (including the CFQ-R 14+). The small number of studies makes it difficult to present clear conclusions regarding the usefulness of existing tools. In turn, new tools that may be used in economic analyses (CFQ-R-8 dimensions) or in individualized assessment of quality of life using a mobile application (Q-Life) seem promising. However, further research on large patient populations is necessary to analyze the measurement properties of all tools.

PMID:39901267 | DOI:10.1186/s12955-025-02338-2

BMC Nurs. 2025 Feb 3;24(1):127. doi: 10.1186/s12912-025-02774-x.

ABSTRACT

BACKGROUND: Cystic Fibrosis (CF) significantly affects the respiratory system, often requiring lung transplantation in advanced stages. This life-saving procedure presents substantial challenges and uncertainties. While there is existing research on support and information needs post-lung transplant from various perspectives, this study aims to specifically address the unique experiences and challenges faced by individuals with CF during both the pre-transplant and post-transplant periods.

METHODS: Twenty-three lung-transplanted individuals with CF participated in this exploratory qualitative study. Data was collected through individual semi-structured interviews and analyzed using inductive content analysis.

RESULTS: Participants faced physical and mental challenges, including fatigue, depression, and anxiety. The waiting period involved isolation, dependence on family, and guilt. Post-transplant, they dealt with relief but also severe pain and adjusted to a new identity. Participants highlighted the importance of taking immunosuppressive medications as prescribed, even though the regimen was complicated and these medications had side effects. Participants stressed the need for earlier and more open dialogue with healthcare professionals and better emotional preparation for the transplant process, including preparedness for pain and previously inadequately addressed concerns such as depression and anxiety.

CONCLUSIONS: This study underscores the significant physical and emotional challenges individuals with CF face during lung transplantation, highlighting the need for comprehensive, person-centered care. Psychological support, effective post-transplant pain management, and early palliative care may be beneficial approaches to improve the patient experience. Nurses can play a pivotal role in this process by ensuring clear communication, managing pain, educating patients on immunosuppressive regimens, and advocating for holistic care.

PMID:39901222 | DOI:10.1186/s12912-025-02774-x

Semin Respir Crit Care Med. 2025 Feb 3. doi: 10.1055/a-2531-1018. Online ahead of print.

ABSTRACT

Sleep disorders that involve circadian rhythm disruption and sleep-disordered breathing (SDB) such as obstructive sleep apnea (OSA) are closely linked to respiratory infections. SDB leads to a proinflammatory state due to intermittent hypoxia, sleep fragmentation, increased oxidative stress, and elevation of inflammatory mediators such as tumor necrosis factor (TNF), interleukin-6 (IL-6), and C-reactive protein (CRP). Furthermore, inflammatory mediator levels correlate with SDB severity, especially in people with OSA. Nocturnal microaspiration, gastroesophageal reflux, and associated comorbidities (e.g. obesity) increase the risk of community-acquired pneumonia, viral infections such as SARS-CoV-2, respiratory complications, and death. OSA has been associated with post-COVID syndrome. It also increases the risk of postoperative complications in both adults and children. Circadian rhythm disorders such as insomnia predispose to immune disorders and increase the risk of infection. Chronic conditions such as bronchiectasis, with or without concomitant cystic fibrosis, can lead to structural sleep changes and increase the risk of OSA due to chronic cough, arousals, aspirations, hypoxia, upper airway edema, and overexpression of proinflammatory cytokines. The protective effect of treatment for sleep disorders against respiratory infection is currently unknown. However, in people presenting with respiratory infection, it is important to test for SDB to prevent complications.

PMID:39900109 | DOI:10.1055/a-2531-1018

Int J Qual Health Care. 2025 Feb 3:mzaf015. doi: 10.1093/intqhc/mzaf015. Online ahead of print.

ABSTRACT

BACKGROUND: Medications are a major cause of harm to patients in hospitals, and several studies have found that they cause approximately 20% of injuries that occur in medical institutions. It was found that the rate of adverse drug events (ADEs) in pediatric hospitalizations ranges from 11 to 40 events per 100 hospitalizations and 1% of cases caused death.Objectives: This is a comparative and retrospective study. The overarching objective is to adapt the Pediatric Trigger Tool (PTT) of the 'Child Health Corporation of America' to pediatric wards in Israel, with the intention of using it to assess the rate of adverse events that occur during medication given in pediatric wards. The study characterized ADEs and examined the ability of the PTT to identify ADEs in relation to those that were voluntarily reported by the staff.

METHOD: This study included internal and surgical pediatric wards at an academic pediatric medical center. The PTT was validated on medical record data from 700 hospitalizations between the years 2015 - 2017. The study also determined, among other things: the stage of drug administration at which the events occurred, the percentage of all events that could have been prevented, the degrees of damage the ADE caused and more.

RESULTS: The Positive Predictive Value (PPV) of the customized tool stands at 16.91%.The study found 108 ADEs in 78 hospitalizations. The ADE rate per 100 hospitalizations was 15.4, the ADE rate per 1,000 drug doses was 3.9, and the ADE rate per 1,000 hospitalization days was 22.8, of which 18.5% were preventable. The category of drugs that led to the highest number of ADEs was painkillers. Those ADEs led to a large number of adverse clinical effects: constipation, hypokalemia, vomiting, and rash. The most common reason for coming to the hospital was suspicion or treatment of a hematologic disease, followed by hospitalization due to a burn. The customized tool found 10.8 times more ADEs than those reported voluntarily-subjectively by the clinicalstaff.

CONCLUSIONS: The study found that, properly adapted, the PTT tool can be used to detectADEs in internal and surgical pediatric wards.

PMID:39898918 | DOI:10.1093/intqhc/mzaf015

Pediatr Pulmonol. 2025 Feb;60(2):e27491. doi: 10.1002/ppul.27491.

ABSTRACT

INTRODUCTION: The Cystic Fibrosis (CF) Foundation guideline for the treatment of pulmonary exacerbations (PEx) does not address empiric antibiotic selection. The primary objective of this study is to characterize how patient-specific microbiological histories are utilized in initial antibiotic selection for CF-related PEx at a pediatric institution. The secondary outcome was to characterize why changes were made to empiric antibiotic regimens.

METHODS: This single-center, retrospective study evaluated individuals aged 1-21 years hospitalized for CF-related PEx at Children's Medical Center Dallas between August 1, 2016 and July 31, 2018.

RESULTS: Among 285 screened hospital encounters, 156 encounters met inclusion criteria. Median age was 12.9 years with a median baseline forced expiratory volume (FEV1) of 84% predicted. Staphylococcus aureus, Pseudomonas aeruginosa, and Stenotrophomonas maltophilia were the organisms most targeted by empiric antibiotics with median months since last growth of 1.5, 9.2, and 5.5, respectively. A difference was observed in median time since last growth for targeted organisms versus those not targeted by the initial antibiotics, but wide overlapping timeframes were noted. Organisms isolated on admission cultures were sensitive to the initial antibiotics regimen in 78.2% of encounters.

CONCLUSION: While variable, patient-specific microbiologic history and time since last growth of historical organisms are taken into consideration when selecting initial antibiotics for the treatment of PEx in children with CF. Expanding initial antibiotic coverage to target microbiological growth histories beyond 1 year prior to a hospital admission did not appear to increase the likelihood of providing coverage for organism(s) isolated on the admission sputum culture in children hospitalized for CF-related PEx.

PMID:39898731 | DOI:10.1002/ppul.27491

Pediatr Pulmonol. 2025 Feb;60(2):e27506. doi: 10.1002/ppul.27506.

ABSTRACT

BACKGROUND: CF R.I.S.E is a program that helps people with Cystic Fibrosis (pwCF) transition from pediatric to adult care. In 2022, we adapted it to CF S.O.B.E in Turkish during a training session. This project aims to present the results of the CF S.O.B.E program.

METHODS: This study included 81 pwCF aged 16-25, divided into two groups: the standard CF S.O.B.E. group (n = 39) and the modified group (n = 42). The standard group received face-to-face education. Both groups participated in online training sessions and received written materials. The knowledge levels were evaluated with Knowledge Assessment Questionnaires (KAQ).

RESULTS: The standard group showed higher post-training scores in "Lung Health and Airway Clearance" and "Equipment Maintenance and Infection Control" (p = 0.014 and 0.002). Modified group showed improvements in all KAQs except "Lung Health and Airway Clearance", "CF-related Liver Disease," "Pancreatic Insufficiency and Nutrition," and "Male Sexual Health." Regarding Pancreatic Insufficiency & Nutrition and CF-related Diabetes, individuals with these conditions demonstrated higher pretest scores than those without these conditions (p = 0.01 and 0.002, respectively). Both groups and their parents reported high satisfaction, and healthcare providers endorsed the program's effectiveness.

CONCLUSION: Our study demonstrated the CF S.O.B.E program's success in enhancing knowledge, disease management skills, and self-confidence among pwCF. While the modified CF S.O.B.E program may be suitable for resource-limited centers, the priority should be to implement the standard program due to its superior outcomes in self-confidence and disease management. This study lays the foundation for incorporating CF S.O.B.E as a standard practice and evaluating its long-term clinical impact.

PMID:39898696 | DOI:10.1002/ppul.27506

Pediatr Pulmonol. 2025 Feb;60(2):e27510. doi: 10.1002/ppul.27510.

NO ABSTRACT

PMID:39898620 | DOI:10.1002/ppul.27510

Pediatr Pulmonol. 2025 Feb;60(2):e27504. doi: 10.1002/ppul.27504.

NO ABSTRACT

PMID:39898600 | DOI:10.1002/ppul.27504

AACE Clin Case Rep. 2024 Oct 4;11(1):32-35. doi: 10.1016/j.aace.2024.09.008. eCollection 2025 Jan-Feb.

ABSTRACT

BACKGROUND/OBJECTIVE: Men with cystic fibrosis (CF) have a high prevalence of low testosterone levels. A recent retrospective study demonstrated a quarter of a cohort of men with CF had serum testosterone levels below 300 ng/dL. The evaluation of hypogonadism is of increasing clinical importance in order to prevent unfavorable outcomes. Herein we present a 31-year-old man with CF and a relatively low serum testosterone value who was found to have an additional unsuspected cause of male hypogonadism.

CASE REPORT: The patient was a 31-year-old man with history of CF who was referred to endocrinology clinic for the evaluation of hypogonadism. Serum testing revealed a total testosterone of 175 ng/mL (296-1377), luteinizing hormone 2.8 mIU/mL (1.2-8.6), and a prolactin of 341 ng/mL (3-13). A brain magnetic resonance imaging was obtained, which revealed a 1 cm hypoenhancing left sellar lesion. He was started on cabergoline. His testosterone increased to 707 ng/dL after a year on cabergoline treatment. His prolactin decreased to 12 ng/mL after a year of treatment. The pituitary adenoma decreased 50% in size 2 years after cabergoline was initiated.

DISCUSSION: The most common etiologies of CF are recurrent infections, chronic inflammation, and glucocorticoid administration, which lead to both hypothalamic-pituitary dysregulation and primary hypogonadism. However, other less common causes of hypogonadism can also be found in CF.

CONCLUSION: We suggest that all men with cystic fibrosis found to have hypogonadism undergo additional evaluation for causes of hypogonadism prior to treatment with testosterone.

PMID:39896950 | PMC:PMC11784604 | DOI:10.1016/j.aace.2024.09.008

OTO Open. 2025 Jan 31;9(1):e70084. doi: 10.1002/oto2.70084. eCollection 2025 Jan-Mar.

ABSTRACT

OBJECTIVE: Individuals with primary ciliary dyskinesia (PCD) frequently report olfactory dysfunction, yet this deficit is poorly documented. The purpose of this study was to characterize the presence and degree of olfactory dysfunction in PCD compared to controls and determine whether certain PCD genes are associated with worse olfaction.

STUDY DESIGN: A prospective cohort study.

SETTING: Tertiary referral center.

METHODS: We administered the University of Pennsylvania Smell Identification Test (UPSIT) to individuals with PCD. Participants were divided into 3 age groups (15-29 years, 30-44 years, and 45+ years) and compared to age- and sex-matched normal controls (n = 2170).

RESULTS: Twenty-nine individuals with PCD (8 males and 21 females) met the criteria (median age: 38 years; interquartile range: 22-47). Only 27.6% of patients with PCD had UPSIT scores within the normosmia range. The UPSIT median scores of each PCD age group were significantly lower than the median scores of the controls (P < .0001 for each age group). UPSIT scores generally worsened with age: mean 33 (mild hyposmia) for 15 to 29 years, 26.8 (moderate hyposmia) for 30 to 44 years, and 20.9 (severe hyposmia) for 45+ years. The most common genes coded were absent inner dynein arm/microtubule disorientation (IDA/MTD) defect (11/24, 45.8%), followed by absent outer dynein arm defect (8/24, 33.3%). The CCDC39 gene (IDA/MTD) was associated with worse olfactory dysfunction.

CONCLUSION: Individuals with PCD have a substantially higher prevalence and degree of olfactory dysfunction compared to age-matched controls. Our study is the first to report greater olfactory dysfunction with age in PCD patients, highlighting an important area for research.

PMID:39896853 | PMC:PMC11783683 | DOI:10.1002/oto2.70084

bioRxiv [Preprint]. 2025 Jan 26:2025.01.24.634776. doi: 10.1101/2025.01.24.634776.

ABSTRACT

Modulator agents that restore cystic fibrosis transmembrane conductance regulator (CFTR) function have revolutionized outcomes in cystic fibrosis, an incurable multisystem disease. Barriers exist to modulator use, making local CFTR gene and cell therapies attractive, especially in the respiratory tract. We used CRISPR to gene-correct CFTR in upper airway basal stem cells (UABCs) and show durable local engraftment into recipient murine respiratory epithelium. Interestingly, the human cells recapitulate the in vivo organization and differentiation of human sinus epithelium, with little expansion or contraction of the engrafted population over time, while retaining expression of the CFTR transgene. Our results indicate that human airway stem cell transplantation with locoregional restoration of CFTR function is a feasible approach for treating CF and potentially other diseases of the respiratory tract.

PMID:39896581 | PMC:PMC11785248 | DOI:10.1101/2025.01.24.634776

Respir Med. 2025 Jan 31:107980. doi: 10.1016/j.rmed.2025.107980. Online ahead of print.

ABSTRACT

BACKGROUND: Patients with cystic fibrosis (CF) use inhaled medicines daily due to respiratory manifestations. However, only 31% of users is inhaling correctly. Digital solutions targeting inhalation could help CF patients improve their technique and thus health outcomes. However, the use of electronic monitoring devices shows a decrease over time. Therefore, the aim of study was to investigate CF patients' preferences for the use of electronic devices on their inhalation technique on a regular basis and reasons behind these preferences.

METHODS: Semistructured interviews were conducted with 11 CF patients from four European countries to understand their disease history and experiences, daily use of inhaler medication, experiences with digital devices to achieve disease control, and expectations of new devices for monitoring inhalation. A conventional content analysis was applied.

RESULTS: CF patients knew their body well due to their lifelong experiences. However, some patients still experienced periods with more symptoms and need for support. Non-app support was preferred. CF patients reported that digital systems should provide high benefits for regular use. Patients differed in their interest in digital systems for inhalation. Such systems were mostly relevant to CF patients starting a new inhaled treatment/inhaler device or during periods in which the disease was out of control.

CONCLUSIONS: CF patients perceived limited value of digital systems to monitor their inhalation and mostly considered them necessary for specific periods. Extensive experience in using inhalers and existing daily routines to manage a high treatment burden appear involved in limited need of such systems.

PMID:39894083 | DOI:10.1016/j.rmed.2025.107980

STAR Protoc. 2025 Jan 31;6(1):103593. doi: 10.1016/j.xpro.2024.103593. Online ahead of print.

ABSTRACT

Here, we present a protocol for the rapid functional screening of gene editing and addition strategies in patient-derived organoids using the deep-learning-based tool DETECTOR (detection of targeted editing of cystic fibrosis transmembrane conductance regulator [CFTR] in organoids). We describe steps for wet-lab experiments, image acquisition, and CFTR function analysis by DETECTOR. We also detail procedures for applying pre-trained models and training custom models on new customized datasets. For complete details on the use and execution of this protocol, refer to Bulcaen et al.1.

PMID:39893642 | DOI:10.1016/j.xpro.2024.103593

Eur J Paediatr Neurol. 2025 Jan 11;54:130-139. doi: 10.1016/j.ejpn.2025.01.004. Online ahead of print.

ABSTRACT

BACKGROUND: Duchenne muscular dystrophy (DMD) is a rare neuromuscular disorder characterized by a progressive decline in muscle function, leading to loss of ambulation, respiratory and cardiac failure, and ultimately death. Improvements in DMD management have increased patient life expectancy; therefore, there is a growing requirement for patients to transfer from paediatric to adult care services. There is also a need for clear recommendations to guide this process.

AIM: To establish international consensus guidelines regarding best practices for transitioning patients with DMD from paediatric to adult care and ensuring continuity of treatment.

METHODS: Consensus statements were developed using the Delphi process and scored using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system. The initiative was led by a steering committee (one non-voting chair and two voting members) who recruited 15 expert panellists to form the consensus group. Following an initial systematic literature search, the consensus group voted in three voting rounds. Round 1 (free-text responses to questions) and Round 2 (importance ranking of statements) were completed using an online survey. Round 3 (voting on final consensus statements) took place during a virtual consensus meeting.

CONSENSUS STATEMENTS: Consensus was reached on 48 statements covering the topics of transition planning, the transition process, post-transfer management, communicating with young people with DMD and supporting them with the transition to adult life.

CONCLUSION: These consensus statements provide guidelines for improving transition practices for young people with DMD and promoting continued care at a comparable standard in adulthood.

PMID:39892019 | DOI:10.1016/j.ejpn.2025.01.004

Ther Adv Respir Dis. 2025 Jan-Dec;19:17534666251317200. doi: 10.1177/17534666251317200.

ABSTRACT

BACKGROUND: Cardiovascular disease (CVD) risks are increasing in people with cystic fibrosis (pwCF). While cholesterol levels were historically low in pwCF, higher levels after initiating highly effective modulator therapy (HEMT) have been reported. Mechanisms are unclear and there is little guidance on screening.

OBJECTIVES: To evaluate serum lipid changes at multiple timepoints after ivacaftor initiation, and to assess current screening practices for CVD risk factors among CF providers.

DESIGN: This was a post-hoc correlative analysis of prospectively collected clinical data and serum samples from the GOAL cohort study. Cross-sectional survey methodology was also employed.

METHODS: We evaluated serum lipids (total cholesterol (TC), low-density lipoprotein (LDL), and high-density lipoprotein (HDL)) at baseline, 3- and 18 months after ivacaftor initiation using samples from the GOAL study biorepository. We also surveyed CF providers across the United States on their CVD risk screening practices.

RESULTS: Fifty GOAL participants' samples were analyzed. Using the repeated measures model, TC significantly varied by visit (p = 0.004), driven by a significant increase from baseline at 3 months (mean difference 9.4 mg/dL). This difference diminished by 18 months. BMI was a significant covariate for TC. No significant differences by visit were detected in LDL or HDL. Seventy-five respondents participated in the survey (response rate 5.6%; 41 adult providers, 18 pediatric providers, and 10 providers caring for both) with 67% reporting no lipid screening policy existed in their center. In the past year, 29% of adult providers prescribed lipid-lowering therapy, 54% started anti-hypertensive medications, and 48% initiated ischemic cardiac evaluations for pwCF.

CONCLUSION: TC significantly increased within 3 months of initiating ivacaftor, but subsequently diminished toward baseline by 18 months. Lipid screening practices among CF providers were variable and providers are increasingly being confronted with managing CVD risk factors. Partnering with primary care providers is likely to become increasingly important in CF care models.

PMID:39891563 | DOI:10.1177/17534666251317200

JAMA Netw Open. 2025 Jan 2;8(1):e2457341. doi: 10.1001/jamanetworkopen.2024.57341.

ABSTRACT

IMPORTANCE: Ultraprocessed foods (UPF), characterized as shelf-stable but nutritionally imbalanced foods, pose a public health crisis worldwide. In adults, UPF consumption is associated with increased obesity risk, but findings among children are inconsistent.

OBJECTIVES: To examine the associations among UPF intake, anthropometric adiposity indicators, and obesity status in Canadian children.

DESIGN, SETTING, AND PARTICIPANTS: In the CHILD Cohort Study, one of the largest prospective, multicenter, population-based pregnancy cohorts in Canada, diet was assessed during the 3-year visit (September 2011 to June 2016), and anthropometric measurements were assessed at the 5-year visit (December 2013 to April 2018). Data analysis was performed between July 1, 2023, and June 30, 2024.

EXPOSURE: Diet intake was assessed using a semiquantitative food frequency questionnaire at 3 years of age. UPFs were identified using the NOVA classification system.

MAIN OUTCOMES AND MEASURES: Anthropometric adiposity indicators were measured at 5 years of age and used to calculate age- and sex-standardized z scores for body mass index (BMI), waist to height ratio, and subscapular and triceps skinfold thicknesses, and obesity, which was defined using BMI z score cutoffs. Multivariable-adjusted regression analyses were used to examine the associations of UPF with adiposity and obesity development, accounting for parental, birth, and early-childhood factors.

RESULTS: Among 2217 participants included in this study, median age at the outcome assessment was 5.0 (IQR, 5.0-5.1) years, and 1175 (53.0%) were males. At 3 years of age, UPF contributed 45.0% of total daily energy intake. UPF energy contribution was higher in males vs females (46.0% vs 43.9%; P < .001). Among all participants, higher UPF intake at 3 years of age was associated with higher anthropometric adiposity indicators at 5 years of age, primarily driven by males. In males, every 10% UPF energy increase was associated with higher adiposity indicator z scores for BMI (β, 0.08; 95% CI, 0.03-0.14), waist to height ratio (β, 0.07; 95% CI, 0.01-0.12), and subscapular (β, 0.12; 95% CI, 0.06-0.18) and triceps (β, 0.09; 95% CI, 0.03-0.15) skinfold thickness and higher odds of living with overweight or obesity (odds ratio, 1.19; 95% CI, 1.03-1.36). No significant associations were observed among females.

CONCLUSIONS AND RELEVANCE: In this cohort study of Canadian children, high UPF consumption during early childhood was associated with obesity development, primarily in males. These findings can inform targeted public health initiatives for early childhood centers and caregiver education programs to reduce UPF intake and prevent obesity.

PMID:39888617 | PMC:PMC11786234 | DOI:10.1001/jamanetworkopen.2024.57341

FEMS Microbiol Lett. 2025 Jan 31:fnaf017. doi: 10.1093/femsle/fnaf017. Online ahead of print.

ABSTRACT

Pseudomonas aeruginosa has increasing clinical relevance and commonly occupies the cystic fibrosis (CF) airways. Its ability to colonize and persist in diverse niches is attributed to its large accessory genome, where prophages represent a common feature and may contribute to its fitness and persistence. We focused on the CF airways niche and used 197 longitudinal isolates from 12 patients persistently infected by P. aeruginosa. We computationally predicted intact prophages for each longitudinal group and scored their long-term persistence. We then confirmed prophage inducibility and mapped their location in the host chromosome with lysate sequencing. Using comparative genomics, we evaluated prophage genomic diversity, long-term persistence and level of genomic maintenance. Our findings support previous findings that most P. aeruginosa genomes harbour prophages some of which can self-induce, and that a common CF-treating antibiotic, ciprofloxacin, can induce prophages. Induced prophage genomes displayed high diversity and even genomic novelty. Finally, all induced prophages persisted long-term with their genomes avoiding gene loss and degradation over four years of host replication in the stressful CF airways niche. This and our detection of phage genes which contribute to host competitiveness and adaptation, lends support to our hypothesis that the vast majority of prophages detected as intact and inducible in this study facilitated their host fitness and persistence.

PMID:39890605 | DOI:10.1093/femsle/fnaf017

J Cyst Fibros. 2025 Jan 30:S1569-1993(25)00001-3. doi: 10.1016/j.jcf.2025.01.001. Online ahead of print.

ABSTRACT

BACKGROUND: CFTR modulator (CFTR-M) therapy has led to improved clinical outcomes amongst people with cystic fibrosis (PwCF) eligible for these therapies. However, there is limited data on their impact on the basic life needs and financial concerns of PwCF.

METHODS: We used data from the Wellness in the Modulator Era (Well-ME) survey, which includes data from 900 PwCF both taking and not taking CFTR-M. We examined self-reported financial well-being over time and changes associated with school or work, financial planning, and costs of living. Descriptive statistics were used to analyze responses.

RESULTS: Most respondents reported no change in financial well-being, but 13 % identified a positive change and 16 % reported a negative change. Positive changes in basic life needs included fewer missed work and school days, while negative changes included medical out-of-pocket costs. Worries about financial problems were reported in 35 % of all respondents and were more common in PwCF who never took CFTR-M or had been taking one and then stopped, in PwCF with lower lung function, and in PwCF with Medicaid insurance.

CONCLUSIONS: These results indicate that for most PwCF, CFTR-M have not affected their basic life needs, and a substantial proportion of PwCF continue to experience financial stress and concerns. Many respondents' financial concerns focused on medical costs and insurance. These data underscore the continued need for CF care teams to address PwCF's financial stress and ability to meet basic life needs, even in the era of improved physical health outcomes due to CFTR-M therapy.

PMID:39890522 | DOI:10.1016/j.jcf.2025.01.001